Scoliosis Associated Cervical Spine Pathologies

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Transcript of Scoliosis Associated Cervical Spine Pathologies

This article appeared in a journal published by Elsevier. The attachedcopy is furnished to the author for internal non-commercial researchand education use, including for instruction at the authors institution

and sharing with colleagues.

Other uses, including reproduction and distribution, or selling orlicensing copies, or posting to personal, institutional or third party

websites are prohibited.

In most cases authors are permitted to post their version of thearticle (e.g. in Word or Tex form) to their personal website orinstitutional repository. Authors requiring further information

regarding Elsevier’s archiving and manuscript policies areencouraged to visit:

http://www.elsevier.com/authorsrights

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Scoliosis-Associated Cervical Spine PathologiesMehmet B. Balio�glu, MDa,*, Akif Albayrak, MDa, Yunus Atıcı, MDa, Mehmet T. Tacal, MDa,

Mehmet A. Kaygusuz, MDa, Can H. Yıldırım, MDb, Miktat Kaya, MDb,Erol Tas‚demiro�glu, MDb, Aytac‚ Akbas‚ak, MDb

aDepartment of Orthopaedics and Traumatology, Disease of the Spine Surgery Group, Metin Sabanci Baltalimani Disease of the Bone Education and

Research Hospital, Rumeli Hisari cd No: 62, 34470 Baltalimani Istanbul, TurkeybDepartment of Neurosurgery, Kafkas University, School of Medicine, Kafkas Universitesi Kampusu Saglik Arastirma ve Uygulama Hastanesi,

36100 Kars, Turkey

Received 2 August 2012; revised 16 September 2013; accepted 2 November 2013

Abstract

Study Design: A total of 126 scoliosis patients admitted to the hospital were screened for concomitant cervical pathologies.Objectives: To investigate the prevalence of cervical spine pathologies and the clinical relevance of magnetic resonance imaging (MRI) inthe evaluation of patients with neuromuscular, congenital, syndromic, and idiopathic scoliosis.Background Summary: With the development of MRI, upper neural axis abnormalities such as syringomyelia and Chiari malformationare increasingly being found in patients with scoliosis, but no report in the literature describes other pathologies in the cervical area seenconcomitant with different scoliosis types.Methods: A total of 126 consecutive patients who were classified as having neuromuscular, congenital, syndromic, and idiopathic scoliosiswere retrospectively evaluated. Data regarding cervical neural axis abnormalities obtained from the MRI studies were analyzed andclassified into each type of scoliosis group.Results: A total of 126 patients with scoliosis were evaluated for hindbrain and cervical spine anomalies. Patients were divided into 4 groupsregarding the type of scoliosis. The cervical spine of all patients was evaluated with MRI and other radiologic methods when needed. The mostfrequently seen pathology was syringomyelia. Other pathologies found included congenital vertebral anomalies, Chiari malformation, arachnoidcyst, atlanto-axial dissociation, split cord, posterior vertebral fusion, vertebral hypoplasia, neurenteric cyst,myelomalacia, dermoid cyst, and decreasein craniovertebral angle. Cervical pathologies were most frequently seen in neuromuscular scoliosis, followed by congenital and syndromic groups.Conclusions: Cervical spinal pathologies vary according to the type of scoliosis. The number of cervical spinal pathologies diagnosed inidiopathic scoliosis patients was least compared with neuromuscular and syndromic groups. The most common pathology was syringo-myelia, followed by congenital vertebral anomalies and cerebral tonsillar hernia. Preoperative MRI scan provides vital informationregarding cervical spinal pathologies encountered in scoliosis patients.� 2014 Scoliosis Research Society.

Keywords: Scoliosis; Cervical spinal pathologies; Magnetic resonance imaging; Neural axis abnormalities; Syringomyelia

Introduction

Magnetic resonance imaging (MRI) scanning in spinaldeformity is often aimed at identifying possible underlying

correctable causes of deformity and conditions that shouldbe treated before surgical correction of the curve itself. Anypatient with an unusual, painful, or rapidly progressingcurve should undergo an MRI, but routine MRI scanningpreoperatively is not indicated in neurologically normaltypical adolescent idiopathic scoliosis (AIS) [1]. Intraspinalanomalies were shown in 20% to 38% of patients withcongenital, infantile, and juvenile scoliosis [2-6]. Theincidence of intraspinal abnormalities reported in patientswith congenital spinal deformity ranged from 15% to 43%[7-13]. Although intraspinal anomalies are rarely found inAIS, the risk increases with left thoracic curves, apical

Author disclosures: MBB (none); AA (none); YA (none); MTT (none);

MAK (none); CHY (none); MK (none); ET (none); AA (none).

*Corresponding author. Department of Orthopaedics and Traumatol-

ogy, Disease of the Spine Surgery Group, Metin Sabanci Baltalimani Dis-

ease of the Bone Education and Research Hospital, Rumeli Hisari cd No:

62, 34470 Baltalimani Istanbul, Turkey. Tel.: þ90 5322521483; fax:

þ90 2123230147.

E-mail address: [email protected] (M.B. Balio�glu).

2212-134X/$ - see front matter � 2014 Scoliosis Research Society.

http://dx.doi.org/10.1016/j.jspd.2013.11.001

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kyphosis, rapidly changing myelodysplastic curves,congenital curves, or any condition with progressiveabnormal neurologic findings [14-16]. Furthermore, un-derlying entities such as Chiari malformations (CM), sy-ringomyelia, cord tethering, or intramedullary spinal cordtumors must be diagnosed because they may present withscoliosis but without neurologic signs. The most commonintraspinal anomaly with scoliosis is Chiari malformationtype I (CM-I) or only syringomyelia [14]. The other mostcommon anomalies are tethered cord, lipoma, dia-stematomyelia, or fatty filum. There has been an increase inCM-I and syringomyelia in patients with spinal deformitysince the beginning of 1990s, when MRI was first used. Itwas shown that 50% to 90% of patients with CM wereassociated with scoliosis and that 15% to 65% of patientswith CM-I had scoliosis [17]. It was reported that scoliosiscould develop alone without syringomyelia with CM-I [18].When correction of scoliosis deformity is necessary, pa-thologies increase the risk of neurological injury by teth-ering the spinal cord [19,20]. Therefore, if there areintraspinal pathologies, a neurosurgical evaluation andtreatment before deformity surgery are necessary becausethere is a risk of neurological deterioration during thedeformity correction [21-24]. The incidence of tetheredcord with CM-I was reported to be 14% [25-28]. Thus, it isadvisable to correct intraspinal anomalies before decom-pression of CM [25,26,29,30].

Proposed indications to perform MRI screening includeearly/juvenile onset, atypical curvature, double thoraciccurve, rapid progression, male gender, abnormal neurologicfindings, and sagittal profile of the spine and the presence ofpain [31,32]. In recent years, studies performed by usingMRIhave shown an increase in the number of osseous spinal andneural pathologies. It has been reported that neurologicsymptoms accompanying osseous spine anomalies man-ifested themselves at the third and fourth decades of lifewithout being noticed previously [33]. Pathologies accom-panying scoliosis in the cervical area include intraspinal andosseous pathologies such as CM, diastematomyelia, syrin-gomyelia, lipoma and lipomeningocele, teratomas, neuren-teric cyst, dermoid cyst, epidermoid cyst, dysmorphic atlas,os odontoideum, C2eC3 fusion, basilar invagination, spinalcanal stenosis, occipitoatlantal instability, atlanto-axialinstability, canal encroachment, myelomalacia, neurofibro-matosis [34], cervical and thoracic hemivertebrae, astrocy-toma, and brain stem tumor [7,32-38].

The most common cervical pathology encountered inpatients with scoliosis is syringomyelia. In patients withidiopathic scoliosis and accompanying syringomyelia,serious neurological complications have been reported aftersurgical interventions performed to correct scoliosiswithout decompressing neurologic tissues before thecorrection operation [39]. Because congenital spineanomalies can be seen in cervical as well as thoracic andlumbar regions, whole spinal axis should be screened,including the cervical region [37]. The associated cervical

anomalies may cause complications during the treatment ofscoliosis, especially during the distraction. Exploration ofneural axis anomalies and osseous pathologies before sur-gery for scoliosis is extremely important.

There are reports in the literature correlated with MRIfindings of whole vertebral axis with scoliosis; and thecurrent study on patients with different types of scoliosisemphasizes the importance of concomitant cervical spinaldisorders using MRI. The objectives of this study were todefine cervical spine pathologies during MRI investigationof the whole spinal axis in patients with scoliosis, and toclassify these pathologies according to the type of scoliosis(ie, infantile and juvenile idiopathic [IS], congenital [CS],neuromuscular [NS], and syndromic [SS]).

Materials and Methods

In this study, patients with scoliosis admitted to thehospital consecutively between 2000 and 2010 with curvesgreater than 20� were examined retrospectively for MRIanalyses of the craniovertebral junction to sacrococcygealregion, and evaluated their cervical spine regions. Magneticresonance images of the cervical pathologies were classi-fied according to scoliosis type. T1W1 and STIR weightedsagittal, and T1W1 coronal and axial images of the cervi-cal, thoracic, lumbar, and sacrococcygeal regions of thespine were examined using 1.5-T magnetic resonanceequipment. A T1W1 sequence was used to evaluate themorphology of the bony structure, vertebral anomalies,osseous tumors, lipoma of the filum terminale, and CM. AT2W1 sequence was used to differentiate among syrinx,tethered cord, or lipomas; and T2W1 and STIR sequenceswere employed in the evaluation of tethered cord, syrinx,and tumors. The radiographic features were CM, syringo-myelia, congenital vertebral anomalies, atlanto-axialdissociation, split cord (diastematomyelia), vertebral hy-poplasia, and a decrease in the craniocervical angle.

Inclusion criteria for the study were curve greater than 20�,definite diagnosis of CS, NS, SS, and onset of IS at less than 10years of age, patients with completely documented data ofmedical records, plain radiograph, andMRI of thewhole spinepreoperatively. Exclusion criteriawere pure kyphosis and onsetof IS at greater than 10 years of age. For the diagnosis of theChiari malformation has been measured as cerebellar tonsilherniation O4 to 5 mm below the foramen magnum [40].Principally patients with intraspinal pathologies were plannedbefore deformity correction; therefore, detection of intraspinalanomalies with MRI was evaluated by neurosurgery.

Results

A total of 126 patients had scoliosis. Patients wereseparated into 4 cohorts according to scoliosis type. Therewere 48 patients (38%) with CS, 44 (34.9%) with NS, 20(15.8%) with SS, and 14 (11.1%) with IS (infantileor juvenile) in each group. Magnetic resonance imaging

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examinations showed cervical pathologies in 22 females(64.7%) and 12 males (35.3%). The mean age was 12.5years (range, 1e35 years) in these groups. Cervical pa-thologies were distributed according to the type of scoliosis(Table 1). When the overall patient group was considered,there were 46 different cervical pathologies in 34 patients(26.9%). The most frequent pathology was syringomyelia,with a 34.7% rate (16 patients); other pathologies werecongenital vertebral anomalies (26%; 12 patients), CM(19.5%; 9 patients), atlanto-axial dissociation (6.5%; 3patients), arachnoid cyst (4.3%; 2 patients), split cord(4.3%; 2 patients), vertebral hypoplasia (2.1%; 1 patient),neurenteric cyst (2.1%; 1 patient), myelomalacia (2.1%;1 patient), dermoid cyst (2.1%; 1 patient), and a decrease incraniovertebral angle (2.1%; 1 patient) (Table 2). Cervicalpathologies were seen most frequently in the NS group,followed by the CS and SS groups (Table 3).

The mean age of patients with NS (44 patients; 34.9%)was 8.4 (range, 1e30 years). Of these, 30 were female(68.2%) and 14 were male (31.8%). The total number ofcervical pathologies was 25 (54.3%), including 7 syringo-myelia, 7 cerebellar tonsillar herniations, 4 congenitalvertebral anomalies, 2 atlanto-axial dislocations, 2 arachnoidcysts, 1 myelomalacia, 1 diastematomyelia, and 1 decreasedcraniocervical angle. Furthermore, 17 of these 44 patientswith NS (38.6%) were operated on for different intraspinalpathologies (12 females and 5 males; mean age, 15.5 years).Moreover, just 3 of these 17 patients (6.8%) required pos-terior spinal surgery and fusion on the cervical area; 2 ofthese 3 patients (4.5%) were CM and 1 (2.2%) was operated

on for a neuroectodermal cyst (Fig. 1). The mean age ofpatients with CS (48 patients; 38%) was 9.8 years (range,1e32 years); 17 were males (35.4%) and 31 were females(64.6%). The CS group consisted of 13 patients (27%) (10females and 3 males), with a mean age of 15 years. Thedistribution of 17 different pathologies (36.9%) was 8congenital vertebral anomalies, 4 syringomyelia, 2 cerebellartonsillar herniations, 1 vertebral hypoplasia, 1 split cord, and1 neurenteric cyst. Furthermore, 5 of these 48 patients(11.3%) underwent surgery by intraspinal pathologies, and 6of these 48 patients (13.6%) were corrected by posteriorinstrumentation. No patients with SS needed surgery forcervical pathologies. Twenty patients (15.8%) with SS wereevaluated; results included were Escobar (2), Marfan (2),spondylo-epiphysial dysplasia (2), Larsen (2), Ganodermaosteodystrophia (2), Pierre Robin syndrome (1), 9P (1),Desbuquios (1), RubinsteineTaybi (1), DiGeorge (1),Eisenmenger (1), Gilbert (1), Morquio (MPS Tip IVA) (1),hereditary motor and sensory neuropathy Tip 4 (1), and 1Pdeletion (1). Four patients (20%) had intraspinal pathologiesupon MRI. All 4 patients with SS were males with a meanage of 14 years. These 4 cervical pathologies (8.7%)included 2 syringomyelia, 1 atlanto-axial dissociation, and 1dermoid cyst. All of the patients with SS did not need sur-gery for cervical pathologies. The mean age of the 14 ISpatients under 10 years of age (11.1%) was 3.2 years (range,1e9 years); 2 of these were male (14.2%) and 12 were fe-male (85.8%). In the IS group, no cervical pathology wasfound upon MRI. Only 2 patients were operated on becauseof an increase in the deformity (Table 4).

No problems were encountered in MRI during anesthesia.Other organ anomalies and orthopedic problems accompa-nied NS, CS, and SS. In particular, patients with spina bifidahad lower extremity motor and sensorial disfunction, boweland bladder disturbance, and neurological impairment withpathological reflex. No neurological disturbances were seenin patients with IS under 10 years of age.

In this study, 24 of 126 patients (19%) underwent neu-rosurgical intervention in different areas of the spine. Ofthese 24 patients, 13 (54%) had myelomeningocele repairs,8 (33.3%) tethered cord releases, 2 (8.3%) suboccipitaldecompression and duraplasty, and 1 (4.1%) excision of thecervicothoracic neuroenteric cyst. When these patientswere evaluated according to their etiologies, 18 (75%) wereNS, 5 (20.8%) were CS, and 1 (4.1%) was SS. However, of

Table 1

Types of scoliosis.

Gender

(female/

male)

Mean age,

years

Patients

per group

Patients with

cervical

pathologies, n (%)

Pathologies,

n (%)

Neuromuscular 12/5 15.3 44 17 (38.6%) 25 (44.6%)

Congenital 10/3 15 48 13 (27%) 17 (30.3%)

Syndromic 0/4 14 20 4 (20%) 4 (7.1%)

Idiopathic 0/0 6 14 0 0

Total/Average 22/12 12.5 (1e35) 126 34 46

Table 2

Frequency of all cervical pathologies.

Pathology Cervical, n (%)

Syringomyelia 13 (28.2%)

Congenital vertebral anomaly 12 (26%)

Chiari malformation 9 (19.5%)

Atlanto-axial dissociation 3 (6.5%)

Arachnoid cyst 2 (4.3%)

Split cord (diastematomyelia) 2 (4.3%)

Vertebral hypoplasia 1 (2.1%)

Neuroenteric cyst 1 (2.1%)

Myelomalacia 1 (2.1%)

Dermoid cyst 1 (2.1%)

Decrease in cranioservical angle 1 (2.1%)

Total 46

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all 126, just 3 (2.3%) with NS required cervical spinalsurgery. In addition, in 13 of these 24 patients, neurosur-gical intervention was required after an average of 4.7 years(range, 6 months to 19.8 years), including posteriorinstrumentation and fusion using pedicle screws (6), ribdistraction device or Vertical Expandable Titanium Ribtechniques (4), growing rods (1), epiphysiodesis (1), andhemivertebra excision (1). According to etiologies, tech-niques included posterior instrumentation and growing rodwith NS (4); posterior instrumentation, hemivertebra exci-sion, growing rod, Vertical Expandable Titanium Rib, andposterior convex epiphysiodesis with CS (6); and posteriorinstrumentation with SS (3). Intraspinal pathologies in pa-tients with 1 SS and 1 CS were operated on using posteriorinstrumentation without an intraspinal approach. Onepatient with spina bifida, CM, and syringomyelia was

operated on by posterior instrumentation 18 years after theinitial operation because of myelomeningocele repairwithout the need for CM treatment (Fig. 2). No patientsdeveloped neurological impairment during or after defor-mity correction.

Discussion

Magnetic resonance imaging is a noninvasive techniquethat provides multiplanar imaging without the use ofionizing radiation. It offers superior soft tissue character-ization, facilitates visualization of complex anatomy, andpermits accurate assessment of the entire neural axis [41].In recent years, examinations performed by means of MRIin the spinal axis showed anatomic and pathologic changes,especially in the cervical region. There have been

Table 3

Distribution of cervical pathologies in all groups.

Neuromuscular Congenital Syndromic Idiopathic Total

Syringomyelia 7 4 2 13

Serebellar tonsillar hernia 7 2 9

Conjenital vertebral anomaly 4 8 12

Atlanto-axial dissociation 2 1 3

Arachnoid cyst 2 2

Split cord (diastemetamyleia) 1 1 2

Neuroenteric cyst 1 1

Myelomalacia 1 1

Dermoid cyst 1 1

Decrease in cranioservical angle 1 1

Vertebral hypoplasia 1 1

Total 25 17 4 0 46

Fig. 1. Chiari malformation-I neuromuscular scoliosis in an 18-year-old boy CM-I. Suboccipital decompression and duraplasty were applied before 2 years

of age owing to due to Chiari malformation-I (A, B). T2e L3 posterior instrumentation and fusion were applied (C, D). Chiari malformation and syringo-

myelia MRI (E, F).

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significant increases in the incidence of CM [32] and sy-ringomyelia [35] in patients with AIS. Although theembryological explanation for variations of the upper cer-vical spine is unclear, abnormalities likely arise duringsomatogenesis as a result of errors in cellular proliferation,migration, differentiation, and segmentation [42]. Severalreports indicated that primary dysfunction of the brain stemand spinal cord contribute to the pathogenesis of IS. After astudy in which scoliosis developed after experimentallyinduced syringomyelia in dogs, Chuma et al. [43] proposedthat dysfunction of the central nervous system maycontribute to the pathogenesis of spinal deformities. Chuet al. [44] found significantly lower cerebellar tonsils inpatients with AIS compared with normal controls. Thediameter of the foramen magnum was considerably longerin patients with AIS than in normal controls, but it has beenproven that this anomaly does not cause changes in cere-brospinal fluid flow velocity. The CM is a condition char-acterized by herniation of the posterior fossa contentsbelow the level of the foramen magnum, and is categorizedinto 3 types based on the degree of herniation. Type I CM is

characterized by caudal descent of the cerebellar tonsilsand is associated with hydromyelic cavitation of the spinalcord in 50% to 76% of patients [45]. In recent years, sincethe advent of MRI, an increasing number of asymptomatic,doubtfully symptomatic, and minimally symptomatic pa-tients with CM-I have been diagnosed. This has resulted incontroversy about the multiple therapeutic strategies indi-cated for these problems. It is believed that herniation ofthe cerebellar tonsils obstructs the cerebrospinal fluid[46,47]. In children, possibly with CS, there may be upperextremity weakness and cranial nerve palsy. Deficiencyafter closing the neural fold leads to undeveloped basi-chondrocranium. It is believed that small and shallowposterior fossa results [46,48,49].

Neural abnormalities in the spine are seen associated withdiastematomyelia, tethered cord, syrinx, CM, Dandy Walkermalformation, intradural lipoma, and arachnoid cyst. In thesesituations, MRI is available as a diagnostic method to un-derstand them. For instability, dynamic cervical MRI can beperformed under observation for spinal cord compression[50]. In a study conducted by Nakahara et al. [31], all spinal

Table 4

Cervical spine pathologies in neuromuscular, congenital, syndromic and idiopathic scoliosis groups.

Neuromuscular Congenital Syndromic Idiopathic

Patients, n (%) 44 (34.9%) 48 (38%) 20 (15.8%) 14 (11.1%)

Patients with cervical pathologies, n (%) 17 (50%) 13 (38.2%) 4 (11.7%) 0

Gender (female/male) 12/5 10/3 0/4 12/2

Age, years (mean [range]) 15.3 (4e30) 15 (5e35) 14 (10e18) 3.2 (1e9)

Pathologies, n (%) 25 (54.3%) 17 (36.9%) 4 (8.7%) 0

Syringomyelia 7 4 2

Cerebellar tonsillar hernia 7 2

Congenital vertebral anomalies 4 8

Atlanto-axial dissociation 2 1

Arachnoid cyst 2

Myelomalacia 1

Diastometamyelia 1 1

Decrease in cranioservical angle 1

Neuroenteric cyst 1

Dermoid cyst 1

Vertebral hypoplasia 1

Fig. 2. Chiari malformation-II neuromuscular scoliosis with spina bifida in an 18-year-old girl who was operated on for myelomeningocele at 1 year of age.

Deficiencies in the lumbar posterior vertebrae, tethered cord, and Chiari malformation (A, B). Progressive scoliosis (C, D). T2-sacroilac posterior instrumen-

tation and fusion were applied (E, F).

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pathologies found in the AIS group, which had 18 patients,were in the cervical region. The incidence of neural axisanomalies with MRI in 472 patients with AIS was 3.8% (18patients); 6 patients had CM-I, 10 had CM-I with syringo-myelia, and 2 had only syringomyelia. With MRI, the re-searchers found significant neural axis abnormalities in malesyounger than 11 years of age and with abnormal superficialabdominal reflex. Even through there was no specific indi-cation for early diagnosis of the neural axis abnormalities,preoperative planning was recommended for routine use.Gupta et al. [35] strongly recommend MRI screening in pa-tients with IS of infantile or juvenile onset, especially forthose who do not have accompanying neurological symptomsand whose curve angle is more than 20�, because of the highincidence of neural axis anomalies. Especially in the 0- to 10-year age group, scoliosis may be the first clue to underlyingneural axis anomalies. In a prospective study, those authorsexamined 34 patients with scoliosis of infantile and juvenileonset; the neural axis anomaly rate was 17.6% (6 of 34 pa-tients). They found CM together with syringomyelia in 1 caseand a brain stem tumor in another. On the other hand, in agroup with 118 patients, they retrospectively evaluated therate of neural axis anomalies to be 20.3% (13 of 64) in patientswith IS, 16% with CS, and 79% with NS. Gupta et al. found 3(50%) intraspinal anomalies in 6 infantile idiopathic scoliosis(IIS) patients, 2 of whom needed neurosurgical interventionfor CM Inoue et al. [32] suggested that patients with IS whoseneurologic status is normal might entail little risk of neuro-logic complications as a result of scoliosis surgery even ifthose patients have a neural axis malformation on MRI.

Magnetic resonance imaging for the preoperative planningof early-onset scoliosis without clinical symptoms with neuralaxis anomalies is important [51]. Incidences of neural axisabnormalities in patientswith ISwere between 2.9%and 37%.Indications forMRI in patientswith scoliosis are considered tobe early onset, atypical curve, double thoracic curve, fastprogression, male gender, and abnormal neurological findings[3,4,14,15,22,23,29,35,40,52-56]. In a prospective trial, Evanset al. [37] performed MRI of the spine and hindbrain in 31patients with scoliosis with an onset between the ages of 4 and12 years. In 8 patients (26%), there was a significant neuro-anatomical abnormality; there were 6 cases of CM-I associ-ated with a syrinx, 1 isolated CM-I, and 1 astrocytoma of thecervical spine. There were no clinical features that couldreliably identify those patients with abnormalities on MRI. Inview of the established risks of surgical correction of scoliosisin the presence of undercompressed syringomyelia and thepossible improvement that may follow decompression of theforamen magnum, MRI of all patients with scoliosis of juve-nile onset should be obligatory [37]. Arnold-CM, syringo-myelia, tethered cord, and neoplasm anomalies have beenreported in approximately 20% of patients with juvenile IS[35,37,57,58]. In patients with IIS, MRI screening is recom-mended because of the high prevalence of secondary intra-spinal anomalies [3,35,37,57]. Lewonowski et al. [57] found50% intraspinal anomaly with MRI (2 of 4 patient) in IIS that

had posterior fossa decompression for syrinx with CM-I.Dobbs et al. [3] found a neural axis abnormality upon MRIin 10 of 46 patients (21.7%). That group included 5 patientswith an Arnold-CM and an associated cervicothoracic syrinx,3with syringomyelia, 1with low-lying conus, and 1with brainstem tumor. Because of the high prevalence of abnormalitiesand the fact that 8 of the 10 patients with abnormal findings onMRI required neurosurgical intervention, a total spine MRIevaluation at the time of presentation is recommended for allpatients with IIS who have a curve measuring 20� or more.Pahys et al. [5] reported that MRI revealed a neural axis ab-normality in 7 of 54 patients (13%)who underwent anMRI. Inthat subset of 7 patients, 5 (71.4%) required neurosurgicalintervention. Tethered cord requiring surgical release wasidentified in 3 patients, CM requiring surgical decompressionin 2 patients, and a small nonoperative syrinx in 2 patients.This study represents the largest evaluation of intraspinalanomalies in IIS to date. In patients with IIS, MRI screeningmay be mandatory for fast growing curve (10�/year or more),change in neurological examination, and planning for thecorrection of the large curve. On the basis of these findings,close observation may be a reasonable alternative to an im-mediate screening MRI in patients presenting with presumedIIS and a curve greater than 20�. According to Fernandes andWeinstein [59], ‘‘curve progression in the patients with early-onset scoliosis should be a major indication.’’

Congenital scoliosis is caused by early embryologic errorsin vertebral column formation. Defining the deformity, pre-dicting the natural history, and applying the correct treatmentcan help ensure successful management. Intraspinal abnor-malities are present in approximately one third of patientswith congenital spine deformities. Curve progression is bestdocumented by measuring identical landmarks on sequentialradiographs.Magnetic resonance imaging is warrantedwhencurve progression is established or when surgical interven-tion is planned [36]. Magnetic resonance imaging of theentire spine and brain stem is mandatory in any child un-dergoing surgical treatment of a congenital spine deformity.The presence of underlying spinal dysraphism is believed tobe upward of 30% in children who have CS [2,6]. Magneticresonance imaging scans also may be studied to look at theanatomy of the deformity. Magnetic resonance imaging beststudies the presence of any cord compression, and this largelyhas replacedmyelography as the imagingmodality of choice.The coronal and sagittal images may help define segmenta-tion and formation defects in the anterior spine. The axialviews also can beviewed to look at the pedicle anatomy of thepatient when contemplating transpedicular instrumentation.Preoperative MRIs can help rule out any coexisting spinaldysraphism. This allows for further imaging of the spinal axisif needed, as well as the ability to clearly image other parts ofthe thorax and abdomen with MRI in patients who havecoexisting organ abnormalities, such as congenital heartdisease or urologic abnormalities [60].

Evaluations with MRI before the surgical treatment ofcongenital spinal deformity have to be made in all children.

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Preoperative MRI may show spinal dysraphism. Spinaldysraphism might be found higher than 30% in patientswith CS [25,60,61]. Intraspinal anomalies are associatedwith hairy patches, sacral dimpling, cavus foot deformity,hyperpigmentation, and/or neurological findings (motor orsensorial deterioration, bladder symptoms, and asymmet-rical abdominal reflex). Complete neurological examinationis needed even if there are no symptoms of intraspinalanomalies. The nonexistence of these symptoms does notnecessarily mean that there are no intraspinal anomalies.Intraspinal anomalies in CS are higher than 38%[6,7,9,10,62,63]. Magnetic resonance imaging shows anyexisting spinal cord compression; myelography is no longerused. There is risk of the neurological injury during thedeformity correction if neural axis abnormalities are notdetected [39]. If the spinal cord is fixed with a bone spiculesuch as diastematomyelia or tethered cord, and is associ-ated with CM and/or syringomyelia or intraspinal tumor,deformity correction may result in spinal cord injury. Curveprogression may result from the spinal dysraphism. If thecurve shows progression and surgery is planned, MRI isrecommended for evaluation of the spinal dysraphism.Using MRI, Prahinski at al. [6] found 9 occult intraspinalanomalies (30%): 5 tethered cord, 4 syringomyelia, 3 li-poma, and 1 diastematomyelia with CS. Two patients(6.6%) needed surgery: 1 with diastematomyelia and 1 withtethered cord. Those authors decided that MRI was bene-ficial in patients with congenital spinal deformity.

According to Belmont et al. [2], 116 patients had CSand a curve that included at least 1 hemivertebra wereidentified. A total of 76 patients had had MRI and wereincluded in the study. The mean age of those patients at thetime of presentation was 4.9 years, and the mean durationof follow-up was 7.7 years. Twenty-nine patients had anisolated hemivertebra and 47 had a complex hemivertebralpattern. Eight of the 29 patients (28%) with an isolatedhemivertebra and 10 of the 47 patients (21%) with acomplex hemivertebral pattern had an intraspinal anomalythat was detected with MRI. Overall, an abnormal findingon the history or physical examination demonstrated anaccuracy of 71%, sensitivity of 56%, specificity of 76%,positive predictive value of 42%, and negative predictivevalue of 85% for the diagnosis of an intraspinal anomaly.Three patients with an isolated hemivertebra and 5 with acomplex hemivertebral pattern underwent neurosurgicalintervention. All 8 patients who underwent neurosurgicalintervention had had detection of an intraspinal anomalywith MRI, whereas only 4 of those patients (2 of whomhad an isolated hemivertebra and 2 of whom had a com-plex hemivertebral pattern) had had an abnormal findingon either the history or the physical examination. Thehistory and physical examination findings are not predic-tive of intraspinal anomalies. Therefore, an MRI evalua-tion of the entire spine should be considered for allpatients with CS, including those with an isolated hemi-vertebra [2].

According to Basu [7], intraspinal abnormalities werefound in 47 patients (37%). These abnormalities weresignificantly more common in patients with congenitalkyphosis (p 5 .0048) and in those with scoliosis resultingfrom mixed and segmentation defects. Scoliosis patientswith cervical and thoracic hemivertebrae had significantlymore intraspinal abnormalities (p 5 .0253) than those withlumbar hemivertebrae. In 64 patients (55%), other organicdefects were found. These defects were more common inpatients with CS resulting from mixed defects (p 5 .002).Cardiac defects were detected in 26%, and urogenitalanomalies in 21% of patients. Magnetic resonance imagingand echocardiography should be an essential part of theevaluation of patients with congenital spinal deformity, andspecial attention should be paid to patients with segmen-tation abnormalities, mixed defects, and kyphosis [7].

According to Suh et al. [63], 41 nonrandomized childrenwith congenital spinal deformities (excluding myelome-ningocele) who underwent complete MRI evaluation werereviewed. Of the 41 congenital spinal deformities, 37demonstrated congenital scoliosis, with failure of formationin 19, failure of segmentation in 4, and mixed defects in 14.The remaining 4 deformities were cases of congenitalkyphosis. Thirteen patients with congenital spine anomalieshad intraspinal abnormalities identified by MRI: tetheredcord in 12 patients, syringomyelia in 3, and dia-stematomyelia in 5. Of the 12 patients with tethered cord, 2had neurologic deficits. Considering a 31% incidence ofintraspinal anomalies, and because clinical manifestationsmay not be initially detectable, MRI is recommended inpatients with congenital spinal deformity as part of theinitial evaluation, even in the absence of clinical findings.

Congenital cervical scoliosis due to a hemivertebra is anunusual spinal deformity. The congenital cervical scoliosiscaused by hemivertebra is a rare but potentially severespinal deformity. The incidence of hemivertebrae in thecervical spine is low compared with those in the thoracicand lumbar regions. The anomaly is often associated withadditional anomalies. The spinal examination also shouldfocus on the cervical spine because of the association ofKlippeleFeil syndrome with CS [60]. The most commonones are anomalies in the cervical spine, such as those ofcongenital block vertebrae (KlippeleFeil syndrome) andCS in the thoracic and lumbar spine [64].

Ozerdemoglu et al. [17] evaluated 105 patients who hadboth scoliosis and syringomyelia, and subdivided them into3 groups: 1) 59 patients without CS or myelomeningocele;2) 20 patients with CS and syringomyelia; and 3) 26 pa-tients with myelomeningocele and syringomyelia. Syrin-gomyelia was diagnosed by surgical exploration (3patients), myelography (4 patients), myelography followedby delayed computed tomography (9 patients), or MRI (89patients). Ten of 16 patients in whom the syrinx was notdiagnosed by MRI had an MRI at later follow-up. Inaddition, only 8 of 105 patients were diagnosed before1980 [17].

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With the aid of MRI, syringomyelia with minimalneurologic deficits can be easily diagnosed in patients withscoliosis. Yeom et al. [38] showed that 20 patients withscoliosis with syringomyelia diagnosed by MRI wereobserved for 6.6 years (range, 2.0e12.6 years) on average.Various factors potentially influencing curve pattern or pro-gression in these patients were then retrospectively reviewed.The location of syrinxwas determined using the sagittal viewof MRI and the size and the side of syrinx using theT1-weighted axial view of MRI. Eight patients (40%) hadsmall syrinx on MRI according to their classification,4 (20%) had a medium one, and 8 (40%) had a large one.Syringes with CM-I tended to be larger than those without amalformation. There was no correlation between syrinx sizeand the size of the major curve of scoliosis. The convex sideof the major curve of scoliosis tended to be on the same sideas the syrinx and as the unilateral neurologic abnormality. Nocorrelationwas found between the location and the size of thesyrinx and the location and size of the major curve of thescoliosis, or between the severity of the neurologic deficit andthe size of the major curve of the scoliosis. In patients underthe age of 10 years at the time of diagnosis of scoliosis andwith a flexible curve, decompression of the syrinx improvedor stabilized scoliosis. In most patients over the age of 10years, surgical treatment of the scoliosis was necessarybecause of the large initial size of the curve or progression ofthe curve even after syrinx drainage. In the series of Yeomet al. syrinx drainage resulted in neurological improvementand/or in a decrease of syrinx size in all patients, as confirmedby MRI. Therefore, patients with atypical scoliosis such asleft thoracic or scoliosis with an unusual appearance, a largecurve at a young age, pain or neurologic deficits, less rotationthan expected, or headaches of unknown etiology shouldundergo an MRI to rule out syringomyelia [22,23,38,65,66].

Neurofibromatosis type 1 (NF-1) is a multisystem diseaseand autosomal dominant genetic disorder. It may manifest asabnormalities of the nervous tissues, bones, soft tissue, andskin. Skeletal abnormalities are common in NF-1. Orthope-dic complications usually appear early, including spinal de-formities such as scoliosis and kyphosis. The cervical spineshould be evaluated at the initial scoliosis assessment. Thecervical abnormalities occur more frequently when scoliosisor kyphoscoliosis is present in the thoracolumbar regionwhere the examiner’s attention is focused on the moreobvious deformity. The most common cervical abnormalityobserved is a severe cervical kyphosis, which in itself ishighly suggestive of the disorder [34]. The incidence ofspinal deformities in associationwithNF-1 varies from2% to36%; scoliosis is the most common. Both heritable and non-heritable factors contribute to the pathogenesis of spinaldeformities in NF-1 patients. Traditionally, the spinal de-formities are classified as dystrophic or non-dystrophic. Thepathophysiology of dystrophic curves is uncertain, but it mayrequire a nonhereditary event such as an adjacent tumor or asecond hit event in local bone cells, leading to the underlyingdysplasia. Dystrophic curves may result in scoliosis,

kyphosis, or frequently, kyphoscoliosis. The dysplastic fea-tures are more commonly associated with deformities of thecervical spine comparedwith other regions of the spine.Mostof the patients with spinal deformities are asymptomatic.When symptoms are present, neck pain is the most commonpresenting concern. In more severe cases, neurologic deficitsmay occur, including nerve root compromise and complete orincomplete spinal cord compromise. However, clinical con-sequences of cervical spine deformities are less marked thanin other areas of the spine because the cord versus canaldiameter is less critical. If there is suspicion of instability,computed tomography and/or flexion-extension MRI areindicated. The most commonly encountered cervical spinedeformity is kyphosis. It may be associatedwith atlanto-axialinstability. Progressive kyphosis is more common thannonprogressive kyphosis. Atlanto-axial instability has beenattributed to laxity of capsular and ligamentous structures.Posterior spinal fusion is recommended in patients withcervical spine instability. The level of instability oftenrequires an occipitocervical fusion [67].

The cervical spine is commonly involved in skeletaldysplasias. It is a good general rule to obtain plain cervicalspine films on all skeletal dysplasia patients and in thosewithabnormalities or marked ligamentous laxity, flexion-extension views. The most common issues are atlanto-axialinstability often associated with odontoid hypoplasia (spon-dylo-epiphyseal dysplasia, Kneist, mucopolysaccharidosis,pseudoachondroplasia, Stickler syndrome, and metatropicdysplasia), cervical kyphosis (diastrophic dysplasia, camp-tomelic dysplasia, and Larsen syndrome), and cervical ste-nosis (achondroplasia, metatropic dysplasia, and Jeunesyndrome) [68-74]. Myelopathy from stenosis, kyphosis, orinstability can be difficult to detect in infants and youngchildren who have not yet myelinated sufficiently to showspasticity. Sleep studies can be helpful. Flexion-extensionMRI can be useful if the radiologist is cooperative, but it iseasy to understand why many are reluctant to do flexion-extension films on anesthetized patients [74]. The skeletalfindings in Loeys-Dietz syndrome (LDS) patient are similarto those found in patients with other connective tissues dis-orders, including Marfan syndrome. Spinal deformities aremost commonly found in the cervical spine. Erkula et al. [75]found cervical spine formation defects or instability in 51%of patients with LDS. Scoliosis was reported in 55% of pa-tients with LDS patients, and dural ectasia was present in67% [76]. Issues involving the cervical spine occurfrequently in children with mucopolysaccharidoses (MPS)and are potentially lives threatening. Spinal cord compres-sion in MPS usually results from a combination of static anddynamic processes: dysplasia of the odontoid process (oftenan os odontoideum), C1 ring hypoplasia, and C1-C2 liga-mentous insufficiency. Glycosaminoglycan accumulationover the odontoid further contributes to stenosis at theoccipital-cervical junction. Atlantoaxial instability isextremely common inMPS IV, to the extent that prophylacticfusion has been advocated for children as young as 4 years. At

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present, spinal cord compression in the cervical spine is themost concerning and consistent pathology in these patients.Multilevel cervical stenosis may be present without insta-bility because of dural thickening in the attenuated forms ofMPS I (I-HS, I-S, Hurler-Scheie, and Scheie) and in MPS VI[77-81]. Down syndrome is a common genetic disorder thathas a significant incidence of spinal pathology. The mostcommon spinal disorder is ligamentous laxity of the cervicalspine that involves either the occiput-C1 level, which causesatlanto-occipital instability, or the C1eC2 level, whichcauses atlanto-axial instability. These areas of cervicalinstability warrant close monitoring for progressive insta-bility or neurological changes that may require restrictionfrom sports or, in certain cases, surgical stabilization. Downsyndrome patients also require close monitoring as they age,because they may also develop acquired degenerativechanges in the cervical spine that may result in myelopathy.Scoliosis may also develop in Down syndrome patients; theincidence is unknown. Two primary areas of the cervicalspine develop instability. The first is between C1 and C2:atlantoaxial instability, which is primarily caused by laxity ofthe transverse ligament of C1 and the joint capsules. Thesecond area of instability occurs at the occipitocervicaljunction and is commonly described as atlanto-occipitalinstability, which is defined by a Powers ratio of greaterthan 1 (anterior instability) and less than 0.55 (posteriorinstability), a basion-axial interval of greater than 12mm andanteroposterior translational motion described by Weiselet al. to be more than 2 mm. Therefore, certain authors haverecommended a flexion-extension MRI scan to evaluateoccipitocervical instability and the presence of spinal cordimpingement. The third cervical pathological problem thatoccurs with Down syndrome patients does not result frominstability, but from premature and advanced subaxialdegenerative changes in the third and fourth decades of lifethat often result in cervical canal narrowing and resultantmyelopathy. Therefore, adult Down syndrome patients whodevelop decreased or altered ambulatory potential should bescreened with MRI to rule out cervical spinal cordcompression [82].

Several abnormalities have been noted in Marfan lumbarspine, including pedicular attenuation and widened inter-pediculate distances. This may result from abnormalities ofgrowth or the presence of dural ectasia. Lumbar pediclewidth and laminar thickness are significantly reduced inMarfan individuals. Those with dural ectasia demonstrateincreased bony erosion of anterior and posterior elementsof lumbosacral spine [83]. Widening of the lumbosacralspinal canal was found in 63% of 57 patients with Marfansyndrome and in none of 57 age- and sex-matched non-Marfan control patients who underwent CT scanning forroutine clinical indications. Bony abnormalities in mildcases consisted of thinning of the pedicles and laminae anderosion of the neural foramina and were generally limitedto L5 and S1. More severe changes were present in 13patients, 2 of whom had associated neurologic signs,

including meningoceles or near total erosion of a pedicle.Presence and severity of vertebral abnormalities wereassociated with no other clinical feature or overall pheno-typic severity. Dural ectasia can be added to the list ofpleiotropic manifestations of Marfan syndrome [84]. Exoticcongenital syndromes include conditions that present withunique challenges for spinal deformity treatment. Patientswith Jeune syndrome have a high rate of proximal cervicalstenosis and should undergo screening with cervical spinefilms at birth. The deformity may be scoliosis, congenital orearly onset, or subtle spine abnormalities, such as C1 ste-nosis in Jeune syndrome. Arthrogryposis may be associatedwith a severe scoliosis and jaw contracture may makeintubation difficult. Larsen syndrome may have early-onsetscoliosis that is rigid and requires early intervention. Cer-vical kyphosis and subluxation may be lethal in these pa-tients and screening radiographs are important. Upperairway abnormalities are an anesthesia concern. Jarcho-Levin syndrome is a thoracic volume depletion deformityresulting from shortness of the thorax, either a spondylo-costal dysostosis variant or spondylothoracic dysplasia.Klippel-Feil deformity is common, so a cervical spine se-ries with flexion-extension laterals should be obtained toassess for stenosis or instability. Screening MRI of thespinal cord should be obtained to rule out abnormalities,especially in spondylocostal dysostosis. Magnetic reso-nance imaging studies of the entire spine are mandatory forsyndromes that have known a central axis nervous systemabnormality, and it is realistic to consider preoperative MRIscan screening of the spinal cord for all patients with exoticcongenital syndromes. Occult spinal cord abnormalitiesthat may increase the risk of neurologic injury duringoperative distraction treatment of the spine in syndromesare often not well characterized in the literature, and MRIscans may detect distal spinal cord tethers, syringomyelia,intradural mass lesions, lateral tethers of the proximalspinal cord, dural ectasia, Arnold-CM, and complex boneecartilage malformations of the spine that may not beevident on plain radiographs [73].

The risks of neurologic complications during and after thecorrection of scoliosis arewidely known. Syringomyeliamaybe a risk factor for neurologic complication during thecorrection of scoliosis, especially during distraction [39].Ozerdemoglu et al. [17] reported that 3 patients (8%) had asignificant deficit after surgery for scoliosis without accom-plishing a surgery for syringomyelia before the correctionoperation. These studies indicate the importance of investi-gating neural axis abnormalities before corrective surgery forscoliosis; however, there is no prospective study revealing therisk of neurologic complications resulting from scoliosissurgery in patients with asymptomatic neural axis malfor-mations. On the other hand, the routine use of MRI iscontroversial, and indications for MRI in scoliosis patientsvary among authors. Some studies recommend treatment ofall syringomyelia before scoliosis correction [17,60,64],whereas others state that routine preoperative MRI is not

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necessary in scoliosis patients unless the patient has aneurologic deficit or pain [1,54,85,86]. In the literature,neurosurgical operations for neural abnormalities in patientswith IS before scoliosis surgerywere 4.5% to 10%.Results ofthe present large, multicenter study demonstrated a 21.7%prevalence of neural axis abnormalities in otherwiseasymptomatic patients with IIS. The high percentage ofneural axis abnormalities in these patients is noteworthybecause 8 of 10 patients who had such abnormalities requiredneurosurgical intervention, compared with 50% of juvenilepatients with neural axis abnormalities, as reported in theliterature [35,57]. Similarly, in the study by Gupta et al. [35],2 of 3 infantile patients with neural axis abnormalitiesrequired neurosurgical intervention. Two of those patientshad CM-I associated with a cervicothoracic syrinx, and thethird patient had diffuse dural ectasia. All 3 patients werebeing treated with a brace for correction of the curve at thetime of the study. In the study by Lewonowski et al. [57], bothpatients with neural axis abnormalities required neurosur-gical intervention to treat CM-I and an associated syrinx. Thereport by Dobbs et al. [3] was believed to be the largestmulticenter study of patients with IIS to date; there was noassociation between abnormal findings on MRI and gender,curve magnitude, curve location, or curve direction. How-ever, because of the 21.7% prevalence of abnormal findingson MRI and the fact that 8 of 10 patients with abnormalfindings required neurosurgical intervention, they recom-mended that MRI of the neural axis be performed at the timeof presentation for all patients with infantile scoliosis whohave a curve measuring 20�, even if the findings of a neuro-logical examination are normal [3]. €Ozt€urk et al. [87]demonstrated that an 8% prevalence of neural axis abnor-malities (20) in 249 patients with adolescent IS; of theseonly 3 required a neurosurgical intervention, yet nonewere inthe cervical region [87]. Twenty patients with scoliosis withsyringomyelia who were diagnosed by MRI were observedfor 6.6 years (range, 2.0e12.6 years) on average. In patientsunder the age of 10 years at diagnosis of scoliosis and with aflexible curve, decompression of the syrinx improved orstabilized scoliosis. In most patients over the age of 10 years,surgical treatment of the scoliosis was necessary because ofthe large initial size of the curve or progression of the curveeven after syrinx drainage. The results of the study of Yeomet al. [38] suggest that early diagnosis and decompression of asyrinx in scoliosis patients especially under the age of 10years is crucial and may decrease the curve size and limitscoliosis curve progression. The current study retrospec-tively evaluated cervical spine in patients with IS under 10years of age and found no neurological abnormalities byMRI. However, there were intraspinal abnormalities in NS(54.3%), CS (35.4%), and SS (20%) patients. Except for 3patientswithNS, neurosurgical interventionwas not requiredin the cervical region.

One issue regarding the MRI study is concern about theuse of general anesthesia in children when necessary.Despite concerns about sedation, the authors consider MRI

to be essential in the detection of occult spinal anomalies.Some authors believe that neurologically asymptomaticpatients with hindbrain and spinal cord abnormalities havelittle risk of neurologic complications from scoliosis sur-gery, even when neural axis malformations are shown onMRI [3,32]. Malviya et al. [88] prospectively evaluated1,140 children (aged 2.96 � 3.7 years) who were sedated.The medical records of children who experienced adverseevents were reviewed. Most children (99%) were monitoredwith pulse oximetry. Of those children, 239 (20.1%)experienced adverse events related to sedation, includinginadequate sedation in 150 (13.2%) and decrease in oxygensaturation in 63 (5.5%). Five children experienced airwayobstruction and 2 became apneic. No adverse event resultedin long-term sequelae. According to another study by thesame authors, quality assurance data were collected pro-spectively for children who were sedated (922) or givengeneral anesthesia (140) for MRI or computed tomography.Reasons for pre-selection of general anesthesia includedpreviously failed sedation (28%), potential for failedsedation (32%), and perceived medical risk (14%). Hyp-oxemia occurred in 2.9% of sedated children, and was morecommon in children classified as ASA III or IV. Sedationwas inadequate for 16% of children and failed in 7% [89].

Especially in terms of anomalies associated with NS,CS, and SS, evaluation with MRI may be important. In thecurrent study on the anomalies associated with scoliosis,surgical treatment was especially necessary in NS. There-fore, the authors believe that the surgeon should know andbe informed about these abnormalities before correctionsurgery. Even if a spinal anomaly is asymptomatic and thepatient is neurologically intact, the behavior of these le-sions after a curve-correction operation is usually unpre-dictable. The authors recommend that MRI be performed inall candidates for scoliosis treatment (except for IS) and anagreement be reached by consensus in a multidisciplinarysetting in which there is a disparity regarding whether anoperation is necessary before the scoliosis correction.

Cervical pathologies may differ according to the type ofscoliosis. With the MRI study in patients with NS, the cer-vical pathology rate was as high as 54.4% compared with theother groups, such as 36.9% in patients with CS, 8.7% withSS, and nonewith infantile or juvenile IS. The most commonpathology is syringomyelia, followed by congenital vertebralanomalies and CM, arachnoid cyst, atlantoaxial dissociation,split cord, vertebral hypoplasia, neurenteric cyst, myeloma-lacia, and dermoid cyst. In the current study, only 3 patientsrequired interventions for the cervical region. These pathol-ogies occurred to a lesser extent in patients with congenitaland syndrome-related pathologies. No patients requiredintervention in the cervical region. No pathologies were seenin the cervical region of IS patients under 10 years of age. Theincidence of intraspinal anomalies in the cervical regionvaries according to the scoliosis etiology. The cervical regionin patients with NS, CS, and SSmust be evaluated withMRI.Evaluation of the cervical region with MRI for IS patients

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under 10 years of agewho have no neurological signs and riskfactors may not be necessary; however, a study examining alarger patient population is needed to better define this.Detailed information about cervical pathologies accompa-nied by scoliosis can be obtained by means of MRI studies.Magnetic resonance imaging evaluation is extremelyimportant for pathologies that cannot be diagnosed by con-ventional methods. A correct diagnosis would prevent manypotential complications from occurring during treatmentoriginating from neural axis malformations.

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